Electrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt β-blocker interruption

Ann Noninvasive Electrocardiol. 2017 Nov;22(6):e12477. doi: 10.1111/anec.12477. Epub 2017 Jun 15.

Abstract

We present a case of a young man with Duchenne muscular dystrophy cardiomyopathy (DMDC) having an implantable cardioverter defibrillator for secondary prevention, who presented with electrical storm shortly after β-blocker interruption. The patient was stabilized and remained free of ventricular arrhythmias soon after reinitiating b-adrenoreceptor antagonists. The present case highlights the importance of sympathetic blockage in patients with DMDC due to existing pathophysiology of excess diastolic Ca2+ leak from sarcoplasmic reticulum as a result of ryanodine receptor dysfunction.

Keywords: cardiomyopathy; duchenne muscular dystrophy; electrical storm; implantable cardioverter defibrillator; β-blocker.

Publication types

  • Case Reports

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use*
  • Adult
  • Arrhythmias, Cardiac / complications*
  • Arrhythmias, Cardiac / diagnosis
  • Defibrillators, Implantable*
  • Electrocardiography / drug effects*
  • Humans
  • Male
  • Medication Adherence*
  • Muscular Dystrophy, Duchenne / complications*
  • Muscular Dystrophy, Duchenne / drug therapy
  • Young Adult

Substances

  • Adrenergic beta-Antagonists